Fetal extraperitoneal rectal perforation presenting after duodenal atresia repair Yew-Wei Tan ⁎ , Sandeep Motiwale Department of Paediatric Surgery, Queen's Medical Centre, Nottingham University Hospital, Nottingham NG7 2UH, UK Received 9 May 2010; revised 11 August 2010; accepted 30 August 2010 Key words: Fetal extraperitoneal rectal perforation; Duodenal atresia; Diagnosis Abstract Fetal extraperitoneal rectal perforation (FERP) is a very rare condition, but prompt diagnosis and appropriate treatment produce overall good outcome. We report the first case of FERP known to be associated with duodenal atresia, which only became clinically apparent after duodenal atresia repair and initially presented a diagnostic difficulty. Our case was successfully treated with a defunctioning colostomy and subsequent excision of the pseudocyst cavity. Proximal atresias can mask the presentation of FERP, and this diagnosis should be considered in cases of neonatal perineal and buttock swelling. © 2010 Elsevier Inc. All rights reserved. Fetal extraperitoneal rectal perforation (FERP) is a terminology first coined by Pitcher et al [1] in 2009. It typically presents in neonates with a gas- or meconium-filled perineal or buttock swelling, leading to a risk of skin rupture, intraperitoneal perforation, and systemic sepsis [1]. On the basis of the 15 reported cases in the literature, it has a good long-term outcome when diagnosed and treated early [1-8]. We present here a case of a newborn known to have duodenal atresia that presented with a concomitant FERP, the latter only became clinically apparent after a duodenoduodenostomy. 1. Case report A 1.76-kg infant girl born by cesarean delivery at 36 +6 weeks gestation was admitted to our neonatal unit with an antenatal diagnosis of duodenal atresia. Polyhydramnios and maternal duodenal atresia were noted. At birth, duodenal atresia was confirmed radiologically. A small discolored left labioperineal swelling was also noted, with a normally placed anus and normal female genitalia. This swelling was clinically thought to be of lymphatic or vascular in origin, but ultrasound of the area was inconclusive. She had a duodenoduodenostomy on day 1 of life and passed meconium on day 2. Within 3 days, her left labioperineal swelling rapidly increased in size (Fig. 1). X-ray and computed tomographic scan of pelvis demon- strated an aerocele with air-fluid level and areas of calcification within the swelling (Fig. 2). A contrast enema confirmed the presence of a communication between the rectum and the cavity but not with the spinal canal (Fig. 3). A spinal ultrasound confirmed the presence of calcification within the extradural spinal canal below the thecal sac. Magnetic resonance imaging showed a small locule of air within the extradural spinal canal at the level of S1 but no definite communication with the cyst. The child remained systematically well with no feature of sepsis or meningism. On day 5, decision was taken to perform a divided sigmoid colostomy to divert feces from ⁎ Corresponding author. Tel.: +44 7786 960622. E-mail address: ywtan80@doctors.net.uk (Y.-W. Tan). www.elsevier.com/locate/jpedsurg 0022-3468/$ – see front matter © 2010 Elsevier Inc. All rights reserved. doi:10.1016/j.jpedsurg.2010.08.063 Journal of Pediatric Surgery (2010) 45, 2447–2449